Department of Pediatrics, Chonnam National University Hospital, Chonnam National University Medical School, Gwangju, Korea. youngcx@hanmail.net

Abstract

Partial anomalous pulmonary vein connection (PAPVC) is a rare congenital abnormal cardiac defect involving the pulmonary veins draining into the right atrium (RA) directly or indirectly by venous connection. Ninety percent of PAPVCs are accompanied by atrial septal defect (ASD). To our knowledge, there is no previous report of PAPVC with ventricular septal defect (VSD) without ASD in Korea, and in this paper, we report the first such case. A 2-day-old girl was admitted into the Chonnam National University Hospital for evaluation of a cardiac murmur. An echocardiogram revealed perimembranous VSD without ASD. She underwent patch closure of the VSD at 5 months of age. Although the VSD was completely closed, she had persistent cardiomegaly with right ventricular volume overload, as revealed by echocardiography. Three years later, cardiac catheterization and chest computed tomography revealed a PAPVC, with the right upper pulmonary vein draining into the right SVC. Therefore, correction of the PAPVC was surgically performed at 3 years of age. We conclude that it is important to suspect PAPVC in patients with right ventricular volume overload, but without ASD.